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Background: Many conditions, including autoimmune disease and idiopathic pulmonary hemosiderosis (IPH), can cause diffuse alveolar hemorrhage (DAH). Little is known about the epidemiology and outcomes in children., Objectives: This retrospective cohort study sought to describe the etiologies and outcomes of DAH in pediatric patients at a tertiary care center., Methods: This study involved review of patient records with diagnostic codes or bronchoscopy reports suggestive of pulmonary hemorrhage at a large children's hospital over 11 years (2010-2020). Patients were included if they met criteria for DAH, defined as bilateral pulmonary infiltrates and at least one of the following: (1) hemoptysis, (2) blood visible on bronchoscopic exam without apparent airway source, or (3) DAH noted on biopsy or autopsy. Infants less than 10 days corrected gestational age were excluded., Results: Seventy-one children with DAH were included in the analysis. Cardiovascular disease was the most common etiology. Bleeding diathesis was common, but all patients had other causes of DAH. Patients with IPH were younger than those with autoimmune disease (p < .001). Most (77%) patients required mechanical ventilation, though this was less common among patients with autoimmune disease. Overall mortality was high (37%) but varied based on underlying etiology; mortality was higher in patients with cardiovascular disease (65%) while no deaths were seen in patients with autoimmune disease or IPH (p = .002). Survivors of DAH who performed pulmonary function tests had normal lung function., Conclusions: DAH frequently causes respiratory failure in children. In our cohort, mortality was highest in patients with cardiovascular disease., (© 2024 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.)

Objective: To describe etiology, clinical profile, treatment and outcome of children with pulmonary hemorrhage., Methods: A chart review of children with pulmonary hemorrhage attending Pediatric Pulmonology services of a tertiary care hospital in North-India was done., Results: Data of 44 children (mean age 59.2 ± 32.1 mo; 28 boys) were included for the study. Possible idiopathic pulmonary hemosiderosis 16 (36.4%), post infectious complications 11 (25%), immune mediated disorders 8 (18.2%), cardiac and vascular disorders 7 (15.9%), and airway pathologies 2 (4.5%) were the etiologies of pulmonary hemorrhage. Treatment options like medications, bronchial artery embolization and surgical resections were offered according to the etiology. Children with idiopathic pulmonary hemosiderosis and those with immune mediated diseases were treated with systemic steroids and steroid sparing agents; the latter group took longer time to respond and had more relapses., Conclusions: Identification of main etiological categories of pulmonary hemorrhage in children could be useful to plan investigations and management of wide range of causes in more practical way.

Idiopathic pulmonary hemosiderosis (IPH) is an extremely rare cause of massive pulmonary hemorrhage in children. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure. We report two cases of IPH children who developed hypoxemic respiratory failure and massive pulmonary hemorrhage. One case of a 10-year-old boy was treated with methylprednisolone pulse therapy (10mg/kg/d) for the first three days and followed by systemic steroid therapy, he successfully decannulated 10days later and discharged with a favorable quality of life. Another case of a 4year-old female child with Down's syndrome diagnosed as IPH for over one year and treated with oral corticosteroids for maintenance therapy. She sudden suffered severe hypoxemia with rapid falls in the hemoglobin level. We applied methylprednisolone pulse therapy (10mg/kg/d) for three days and other supportive therapies, the girl survived through complicated with oxygen dependence. We suggest that methylprednisolone pulse therapy provides a chance of recovery and survival for patients with IPH at the acute phase, even if accompanied by severe pulmonary hemorrhage., (Copyright © 2017 Elsevier Inc. All rights reserved.)

This article reports 4 girls with clinical manifestations of recurrent cough and anemia. The age of onset was less than 4 years, and three of them had shortness of breath. None of them had acute hemoptysis. All the girls had positive results of hemosiderin test for bronchoalveolar lavage fluid. As for imaging examination, 3 patients had ground-glass opacity, and 1 had interstitial change. Three girls were given the treatment for idiopathic pulmonary hemosiderosis and had no response. Selective bronchial arteriography was performed for the 4 girls and found bronchial artery to pulmonary circulation shunt (BPS). After they were diagnosed with BPS, they were given transcatheter embolization. The girls were followed up for half a year after surgery, and none of them was readmitted due to "cough and anemia". BPS manifests as abnormal shunt between the bronchial artery and the pulmonary artery/vein and has unknown causes. It is rare in children and should be considered for children who were thought to have idiopathic pulmonary hemosiderosis and had poor response to corticosteroid therapy.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) in pediatric patients. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure with associated multiple organ failure often occurs. We report the case of an 11-year-old girl who developed severe hypoxemic respiratory failure and pulmonary hemorrhage. Extracorporeal membrane oxygenation (ECMO) was instituted on the next day because medical treatment and mechanical ventilation failed to maintain oxygenation. She was successfully decannulated 5 days later without ECMO-related complications. Four months later, she was intubated again and the diagnosis of IPH was made by open lung biopsy. She was treated with systemic steroid therapy and discharged smoothly. We suggest that ECMO provides a chance of recovery and survival for patients with IPH, even if accompanied by severe pulmonary hemorrhage.

A 35 year-old man was admitted to our hospital because of cough, wheezing, and paresthesia of the right upper extremity. He demonstrated marked eosinophilia, bronchial asthma, and mononeuritis multiplex. We diagnosed Churg-Strauss syndrome. Serum MPO-ANCA was elevated to 189 U/ml. Chest high-resolution computed tomography showed panlobular ground-glass attenuation in both lungs. BALF showed bloody fluid, and TBLB revealed findings consistent with eosinophilic pneumonia. Lung biopsy by VATS revealed eosinophilic pneumonia, pulmonary vasculitis, capillaritis, and hemosiderosis. The patient recovered after treatment with prednisolone and cyclophosphamide. We concluded that alveolar hemorrhage due to pulmonary capillaritis could be a complication in cases of Churg-Strauss syndrome.

Objective: To determine clinical features and outcome in hospitalized patients with acute lung injury secondary to diffuse pulmonary hemorrhage (DPH)., Patients and Method: Eight adult patients suffering DPH and acute lung injury were undergone to diagnosis procedure and following., Results: Two cases of idiopathic pulmonary hemosiderosis, two cases of systemic lupus erythematous, two cases of Goodpasture's syndrome, one case of idiopathic rapidly progressive glomerulonephritis and one of periarteritis nodosa were diagnosed. Treatment was methylprednisolone in all cases; besides, three cases required ventilatory support, and one immunosuppressive therapy, plasmapheresis and hemodialysis. One patient died and three of them suffered relapse with satisfactory outcome., Conclusion: We remark the presence of acute lung injury as an initial feature of DPH in our experience and the importance of a prompt diagnosis of the underlying disease.

In November 1994, private physicians and public health officials in Cleveland, Ohio, and CDC reported a cluster of eight cases of acute pulmonary hemorrhage/hemosiderosis that had occurred during January 1993-November 1994 among infants in one area of the city. Two additional cases were identified in December 1994. All 10 infants lived within seven contiguous postal tracts in eastern metropolitan Cleveland. Pulmonary hemorrhages recurred in five of the infants after they returned to their homes shortly after hospital discharge; one infant died as a result of pulmonary hemorrhage. This report summarizes the findings of the follow-up investigation, including a case-control study and an assessment by the county coroner of cases of infant death. These findings documented an association between acute pulmonary hemorrhage/hemosiderosis in this cluster of cases and mold growth in their water-damaged homes.

Diffuse pulmonary hemorrhage (DPH) is a syndrome characterized by the presence of widespread hemorrhage from the pulmonary microvasculature leading to hemoptysis, iron deficiency anemia, and a chest radiography showing bilateral airspace consolidation. Diagnostic imaging consists primarily of chest radiography, but CT and MR imaging may be helpful in selected cases. There are many causes of DPH, and the differential diagnosis and diagnostic approach depend on whether the patient is immunocompetent or immunocompromised. This review summarizes the clinical, pathologic, and imaging features of DPH and the treatment of its more common causes.

We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the usefulness of liposteroid in the management of refractory IPH.

Circulating immune complexes occurred during pulmonary hemorrhage in a pregnant patient with idiopathic pulmonary hemosiderosis, an association not previously reported. The patient required mechanical ventilation, but recovered; after a prolonged hospitalization, she was delivered of a healthy infant without further complications.

360 consecutive bronchoalveolar lavage fluids (BAL) were studied using semi-quantitative method described by GOLDE (GOLDE score). 44 hemosiderosis were detected (12%). Most of them corresponded to massive alveolar hemorrhage (AH). Depending on pathological contexts, we distinguished three main groups of AH frequency: a group of high frequency (around 40%), an intermediate group (10%) and a low frequency group with less than 5%. Matching cytological results with clinical and radiological data, revealed that AH was often too late diagnosed because inconstancy of typical clinical and radiological signs. Even with a variable prognosis related to different physiopathological mechanisms, AH evolution is unpredictable. The authors suggest thus BAL cytological examination may systematically include a research of hemosiderosis in AH high frequency groups.

In the first part of this article the clinical and paraclinical criteria of intra-alveolar haemorrhages are reviewed. The principal signs and symptoms are dyspnoea, haemoptysis, anaemia and bilateral alveolar and interstitial opacities. Computerized tomography of the chest shows a non-systematized alveolar filling. In the second part, the cytological and histological criteria are considered; they are provided by examination of the alveolar lavage fluid and by lung biopsy. Finally, the main elements in the physiopathology and causes of these haemorrhages are successively described.

Diffuse pulmonary hemorrhage is a syndrome consisting of hemoptysis, anemia, and air-space consolidation. The radiologic appearance is non-specific. Pulmonary hemorrhage may be due to a number of different causes. The differential diagnosis and the diagnostic approach are different in the immunologically intact host as compared with the immunocompromised host. This article reviews the main diagnostic considerations in diffuse pulmonary hemorrhage.

We have reviewed the alveolar hemorrhage (AH) syndromes, defined as immune or idiopathic disorders associated with diffuse microvascular hemorrhage into the acinar portion of the lung. The disorders that are most often associated with AH include antibasement membrane antibodies (ABMA) disease, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus, systemic vasculitides, and idiopathic rapidly progressive glomerulonephritis. An approach to the recognition, diagnosis, and treatment of the AH syndromes has been outlined and several illustrative case studies have been presented. Recognition of AH is not usually difficult, but does require a high index of suspicion, since many disease processes may give rise to hemoptysis with infiltrates on chest roentgenogram. Recognition of AH is aided by careful clinical and laboratory assessment for evidence of extrapulmonary disease; simple hematologic studies such as sequential hemoglobins and iron studies; and measurement of carbon monoxide uptake by the lungs. Early recognition of AH may decrease the likelihood of respiratory failure and end-stage renal disease. The specific etiology of AH is usually determined by clinical examination, serologic assay for ABMA, and percutaneous renal biopsy by immunofluorescence. Open-lung biopsy is required in a minority of cases. High-dose pulse methylprednisolone appears to effectively control AH of diverse etiology. Combined plasma exchange and immunosuppression controls AH in ABMA disease and is the treatment of choice in this disorder. Cyclophosphamide is used for Wegener's granulomatosis, and sometimes in systemic necrotizing vasculitis, in an attempt to prevent irreversible damage to the kidneys.

Serial changes in carbon monoxide uptake (KCO or DLCO/VA) were used to monitor episodes of pulmonary capillary haemorrhage in Goodpasture's syndrome (13 cases), immune complex nephritis (three cases) and idiopathic haemosiderosis (one case). Air-space shadowing on the chest X-ray (on a scoring system 0 to 12) was assessed in the light of the changes of KCO. In 14 out of 27 episodes of bleeding, the rise and fall of KCO was matched in time by the appearance and disappearance of air-space shadowing on the chest X-ray. In six episodes the chest X-ray remained normal despite a rise of KCO. In two cases air-space shadowing appeared up to 48 h after the rise of KCO. On five occasions chest X-ray abnormalities preceded the rise of KCO but chest infection or fluid overload accounted for three of these. In cases with suspected pulmonary capillary haemorrhage, measurements of carbon monoxide uptake will provide additional information and will assist in the interpretation of the chest X-ray.

Diffuse pulmonary hemorrhage is an uncommon condition that is difficult to differentiate radiographically from diffuse pneumonia or pulmonary edema. The diagnosis should be suspected when a patient has even mild hemoptysis or has one of the diseases known to be associated with diffuse pulmonary hemorrhage. This paper reviews the clinical and radiographic features of diffuse pulmonary hemorrhage and presents a classification scheme depicted as a Venn diagram formed by four overlapping circles representing pulmonary hemorrhage, renal disease, immune complex disease, and antiglomerular basement membrane (anti-GBM) disease. This scheme results in six categories of pulmonary hemorrhage: associated with glomerulonephritis and anti-GBM antibody; associated with renal disease without demonstrable immunologic abnormalities; associated with glomerulonephritis and immune complex disease; associated with immune complex disease without renal disease; associated with anti-GBM antibodies without renal disease; without associated immunologic or renal abnormality. Examples of these disorders are illustrated. Improved clinical-radiographic correlation may lead to earlier diagnosis and treatment of diffuse pulmonary hemorrhage and its causes.